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Glucocorticoid and cyclosporine refractory adult onset Still’s disease successfully treated with tocilizumab

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Abstract

We report a 29-year-old Japanese woman with disseminated intravascular coagulation (DIC) and adult onset Still’s disease (AOSD). Her disease was refractory to high-dose glucocorticoids, two courses of steroid pulse therapy, and addition of cyclosporine (3.5 mg/kg/day). The serum interleukin-6 level was markedly elevated. Therefore, we administered an anti-interleukin-6 receptor antibody (tocilizumab, 8 mg/kg fortnightly), which dramatically improved her symptoms and the levels of acute-phase proteins. In addition, rapid tapering of the glucocorticoid dose was possible. Four months later, she was maintained on tocilizumab infusion once a month with low-dose steroid therapy. Cyclosporine is one of the first-line immunosuppressants for AOSD, especially when associated with DIC, hepatic failure, or hemophagocytic syndrome. In patients with cyclosporine-resistant AOSD, tocilizumab may be another useful option.

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Disclosures

Dr. Iwamoto and Minota have received royalties from Chugai Pharmaceutical Company, Ltd., Tokyo, Japan (less than $10,000). The other authors have disclosed no conflicts of interest.

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Authors and Affiliations

  1. Division of Rheumatology and Clinical Immunology, Jichi Medical University, Yakushiji 3311-1, Shimotsuke-shi, Tochigi, 329-0498, Japan

    Kazuko Matsumoto, Takao Nagashima, Shino Takatori, Yuta Kawahara, Masaki Yagi, Masahiro Iwamoto, Hitoaki Okazaki & Seiji Minota

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  1. Kazuko Matsumoto
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  2. Takao Nagashima
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  3. Shino Takatori
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  4. Yuta Kawahara
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  5. Masaki Yagi
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  6. Masahiro Iwamoto
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  7. Hitoaki Okazaki
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  8. Seiji Minota
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Correspondence to Takao Nagashima.

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Matsumoto, K., Nagashima, T., Takatori, S. et al. Glucocorticoid and cyclosporine refractory adult onset Still’s disease successfully treated with tocilizumab. Clin Rheumatol 28, 485–487 (2009). https://doi.org/10.1007/s10067-009-1097-z

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  • DOI: https://doi.org/10.1007/s10067-009-1097-z

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